CLINICAL CHARACTERISTICS OF DIFFERENT TYPES OF CRANIAL DYSTONIA: AN OBSERVATIONAL STUDY.

Abstract

Background:  Cranial dystonias have complex phenomenology and heterogenous etiology. Large prospective observational studies would help us in a better understanding and management of this complex disorder.

Objectives: To study the clinic-etiological profile of patients of cranial dystonia attending a movement disorder clinic of a tertiary care teaching institution.

Methods: All the consecutive cranial dystonia patients from October 2017 to September 2019 were enrolled and classified according to the consensus update on phenomenology and classification of dystonia.

Results: Total number of patients with cranial dystonia were 96, with 42(43.8%) females and 54 (56.3%) male, with M: F=1.28:1. Mean age of onset was 42.36± 21 years with mean duration of symptoms was 3.38 ± 4.05 years Oromandibular dystonia was the most common type of cranial dystonia (45.8%) followed by Meige syndrome (28%) and blepharospasm (26%). Meige  syndrome started as blepharospasm in 85.1%(p<0.001).  Jaw opening dystonia(JOD) was overall  the most common (42.3%) type of oromandibular dystonia. In 9.8% idiopathic cases and 28.9% non-idiopathic cases, dystonia started from extracranial region (p=0.02). JOD (51.1%) was the most common type of oromandibular movement abnormality in non-idiopathic cranial dystonia where-as in idiopathic cases jaw closure dystonia (JCD) and oro-facial dystonia (OF), (15.7% each) were the most (p<0.001).

Conclusion: In present study we observed thatcranial dystonia started one decade earlier than western population. Cranial dystonia with simultaneous extracranial involvement, and starting from the oromandibular region was more likely to be non-idiopathic aetiology.